Eosinophilic granulomatosis with polyangiitis: an overview.

摘要: Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disorder, belonging to the small vessel ANCA-associated vasculitis, defined as eosinophil-rich and necrotizing granulomatous inflammation often involving respiratory tract, vasculitis predominantly affecting medium-sized vessels, associated asthma eosinophilia. EGPA pathogenesis not well known: HLA-DRB1*04 *07, HLA-DRB4 IL10.2 haplotype of IL-10 promoter gene are most studied genetic determinants. Among acquired pathogenetic factors, exposure different allergens, infections, vaccinations, drugs silica have been involved. Eosinophils characteristic cells in studies demonstrated their role effector immunoregulatory cells. considered disease prevalent activation Th2 cellular-mediated inflammatory response but also humoral immunity plays an important role. A link between B T responses may explain features. typically develops into three sequential phases: allergic phase, distinguished by occurrence asthma, rhinitis sinusitis, eosinophilic which main pathological finding organ infiltrations (e.g. lungs, heart gastrointestinal system) vasculitic characterized purpura, peripheral neuropathy constitutional symptoms. ANCA (especially pANCA anti-MPO) present 40-60% patients. An elevation IgG4 frequently found. Corticosteroids cyclophosphamide classically used for remission induction, while azathioprine methotrexate therapeutic options maintenance. B-cell depletion rituximab has shown promising results induction.